Managing Rheumatic Fever: Diagnosis, Treatment and Long-Term Effects.

What is Rheumatic Fever?

Rheumatic fever is an inflammatory disease that can develop as a complication of untreated or inadequately treated streptococcal throat infection (strep throat). It primarily affects children between the ages of 5 and 15 but can also occur in adults. The disease can lead to serious complications, including permanent damage to the heart, known as rheumatic heart disease (RHD).

Causes of Rheumatic Fever:

Understanding the causes of rheumatic fever is essential for its prevention and early management.

► Streptococcal Throat Infection (Strep Throat):

The primary cause of rheumatic fever is an infection caused by Group A Streptococcus (GAS) bacteria, which leads to strep throat or, in some cases, scarlet fever. If left untreated, the body’s immune response to this bacterial infection can mistakenly attack its own tissues, triggering an inflammatory reaction characteristic of rheumatic fever.

► Autoimmune Reaction:

Rheumatic fever is not directly caused by the bacteria itself but by the body’s immune response to the infection. When the immune system fights the streptococcal bacteria, it produces antibodies. However, these antibodies may mistakenly attack healthy tissues in the heart, joints, skin, and brain due to molecular mimicry—where bacterial proteins resemble those of human tissues. This autoimmune response leads to inflammation and damage.

► Genetic Predisposition:

Some individuals are more genetically susceptible to developing rheumatic fever. A family history of the disease increases the likelihood of an exaggerated immune response to streptococcal infections. Certain genetic markers have been associated with a higher risk of developing the condition, suggesting that heredity plays a role.

► Poor Access to Healthcare and Delayed Treatment:

Inadequate access to medical care can contribute to the development of rheumatic fever. If strep throat is left untreated or antibiotics are not administered properly, the risk of developing the disease increases significantly. This is especially common in underprivileged or remote areas where medical facilities and awareness about streptococcal infections are limited.

► Recurrent Streptococcal Infections:

Individuals who experience frequent episodes of strep throat without proper treatment are at a higher risk of developing rheumatic fever. Repeated exposure to Group A Streptococcus bacteria increases the chances of an abnormal immune response, leading to progressive inflammation and tissue damage.

► Environmental Factors:

Living in overcrowded or unhygienic conditions can increase the spread of streptococcal infections, thereby raising the risk of rheumatic fever. Poor sanitation, lack of clean drinking water, and close-contact living situations make it easier for bacteria to spread from person to person.

► Age and Gender Factors:

Rheumatic fever primarily affects children between the ages of 5 and 15, as their immune systems are still developing. Although it can occur in adults, it is much rarer. Additionally, some studies suggest that females may be more prone to developing the disease than males, although the reasons are not yet fully understood.

Symptoms of Rheumatic Fever:

Recognizing the symptoms early is crucial to prevent severe complications, such as rheumatic heart disease. Below are the key symptoms of rheumatic fever:

◆ Fever:

A persistent high fever is one of the earliest symptoms of rheumatic fever. The fever often exceeds 101°F (38.3°C) and may be accompanied by chills and general malaise.

◆ Painful and Swollen Joints (Arthritis):

One of the hallmark symptoms is migratory arthritis, which means that the pain and swelling move from one joint to another. The knees, ankles, elbows, and wrists are commonly affected. The inflammation may last for a few days in one joint before shifting to another.

◆ Chest Pain and Heart Problems (Carditis):

Inflammation of the heart (carditis) is a severe complication of rheumatic fever. Symptoms include chest pain, shortness of breath, rapid heartbeat, and fatigue. In some cases, a heart murmur may develop, indicating damage to the heart valves.

◆ Skin Rash (Erythema Marginatum):

A rare but distinct symptom of rheumatic fever is a pink, ring-shaped rash that appears on the trunk and limbs. The rash is usually painless and does not itch.

◆ Uncontrolled Movements (Sydenham’s Chorea):

Some individuals, especially children, develop Sydenham’s chorea, a neurological disorder characterized by involuntary, jerky movements of the face, hands, and feet. These movements may worsen with stress and subside during sleep.

◆ Small, Painless Nodules Under the Skin:

In some cases, firm, painless lumps called subcutaneous nodules develop under the skin, particularly near joints. These nodules are uncommon and usually occur in individuals with severe heart involvement.

◆ Fatigue and Weakness:

People with rheumatic fever often feel extremely tired and weak. This is due to the systemic inflammation affecting multiple organs and tissues.

◆ Sore Throat and Difficulty Swallowing:

Since rheumatic fever is a complication of untreated strep throat, a prior history of sore throat, difficulty swallowing, and swollen tonsils may be present.

◆ Behavioral Changes:

Some individuals experience mood swings, irritability, and difficulty concentrating, which are associated with neurological involvement in Sydenham’s chorea.

Diagnosis of Rheumatic Fever:

Early and accurate diagnosis is crucial for effective management and prevention of long-term sequelae.

Clinical Criteria for Diagnosis:

The diagnosis of rheumatic fever is based on the Jones Criteria, initially established in 1944 and periodically updated by the American Heart Association (AHA). The latest modification includes both major and minor criteria, along with evidence of a preceding streptococcal infection.

Major Criteria:

1. Carditis – Inflammation of the heart, affecting the endocardium, myocardium, or pericardium, often presenting as murmurs, heart enlargement, or congestive heart failure.
2. Polyarthritis – A migratory inflammation of large joints, typically affecting the knees, ankles, elbows, and wrists.
3. Chorea (Sydenham’s Chorea) – Neurological symptoms characterized by involuntary, jerky movements of the face, hands, and feet.
4. Erythema Marginatum – A rare skin rash featuring pink rings with clear centers, commonly found on the trunk and limbs.
5. Subcutaneous Nodules – Firm, painless nodules under the skin, often found over bony prominences.

Minor Criteria:

★ Fever – Typically between 38.2°C and 39.5°C (100.8°F – 103.1°F).

★ Arthralgia – Joint pain without inflammation.

★ Elevated Acute Phase Reactants – Increased erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), or leukocytosis.

★ Prolonged PR Interval – Abnormal electrocardiogram (ECG) findings indicating heart conduction delay.

Laboratory and Diagnostic Tests:

To confirm rheumatic fever, laboratory and imaging tests are employed to support clinical findings.

► Evidence of Streptococcal Infection:

• Throat Culture – Identifies Group A Streptococcus (GAS) in the throat.
• Rapid Antigen Detection Test (RADT) – Quick method to detect GAS antigens.
• Anti-streptolysin O (ASO) Titer – Elevated levels suggest a recent streptococcal infection.
• Anti-DNase B Test – An alternative marker for previous streptococcal infection.

► Electrocardiogram (ECG):

• Detects prolonged PR interval and other conduction abnormalities.

► Echocardiography:

• Identifies valvular damage, pericardial effusion, or myocardial involvement.

► Blood Tests:

• Complete Blood Count (CBC) – May show leukocytosis.
• Erythrocyte Sedimentation Rate (ESR) and C-reactive Protein (CRP) – Indicators of systemic inflammation.

Differential Diagnosis:

Rheumatic fever shares symptoms with several conditions, requiring careful differentiation. These include:

★ Juvenile idiopathic arthritis (JIA).

★ Infective endocarditis.

★ Systemic lupus erythematosus (SLE).

★ Reactive arthritis.

★ Viral myocarditis.

Treatment of Rheumatic Fever:

Effective treatment aims to eliminate the underlying infection, manage inflammation, and prevent recurrence.

► Antibiotic Therapy:
The cornerstone of rheumatic fever treatment is antibiotic therapy to eradicate Group A Streptococcus bacteria. Penicillin is the preferred antibiotic, administered as either a single intramuscular injection of benzathine penicillin G or a 10-day course of oral penicillin. For patients allergic to penicillin, alternatives such as erythromycin or azithromycin can be used.

► Anti-Inflammatory Medications:
Since rheumatic fever causes inflammation in joints, skin, heart, and the nervous system, anti-inflammatory medications are crucial for symptom relief.

★ Aspirin: High-dose aspirin is commonly used to control fever and arthritis symptoms.

★ Corticosteroids: In severe cases, especially with carditis, corticosteroids like prednisone may be prescribed to reduce inflammation.

► Management of Carditis:
Patients with rheumatic fever affecting the heart require close monitoring. Severe cases may necessitate hospitalization. In some instances, long-term management includes medications such as diuretics and beta-blockers to support heart function.

► Supportive Care:

· Bed Rest: Patients with active symptoms, particularly carditis, are advised to rest until inflammation subsides.

·  Hydration and Nutrition: A balanced diet rich in vitamins and minerals supports recovery.

► Secondary Prevention:
Preventing recurrence is critical, as repeated episodes increase the risk of long-term complications. Long-term antibiotic prophylaxis is recommended, often continuing into adulthood for individuals with heart involvement.

★ Monthly intramuscular penicillin injections: The most effective method for prevention.

★ Oral penicillin or erythromycin: Alternative for those unable to tolerate injections.

► Surgical Intervention:
In advanced cases where rheumatic heart disease has led to valve damage, surgical procedures such as valve repair or replacement may be necessary.

Prevention of Rheumatic Fever:

Since there is no specific cure for rheumatic fever, prevention remains the best approach to managing the disease.

Primary Prevention:

The first and most effective strategy to prevent rheumatic fever is to prevent the initial streptococcal throat infection or treat it promptly. This is known as primary prevention, which includes the following measures:

◆ Early Detection and Treatment of Strep Throat:

• Identify and diagnose streptococcal throat infections early.
• Seek medical attention if symptoms such as sore throat, fever, swollen lymph nodes, and difficulty swallowing appear.
• Laboratory tests, including rapid antigen detection tests and throat cultures, can confirm the presence of Streptococcus pyogenes.

◆ Proper Use of Antibiotics:

• A full course of antibiotics, usually penicillin or amoxicillin, should be taken as prescribed by a healthcare professional.
• Avoid stopping antibiotic treatment early, even if symptoms improve, to ensure complete eradication of the bacteria.

◆ Good Hygiene Practices:

• Wash hands frequently with soap and water to prevent the spread of infections.
• Avoid close contact with individuals who have strep throat.
• Cover the mouth and nose when sneezing or coughing.

◆ Strengthening the Immune System:

• Maintain a balanced diet rich in vitamins and minerals to enhance immunity.
• Get adequate sleep and engage in regular physical activity.

Secondary Prevention:

For individuals who have had rheumatic fever, secondary prevention is crucial to prevent recurrences, which can increase the risk of heart damage. This involves:

★ Long-term Antibiotic Prophylaxis:

• Patients with a history of rheumatic fever may require long-term antibiotic treatment to prevent recurrent infections.
• The most commonly prescribed antibiotic is benzathine penicillin, given as an intramuscular injection every three to four weeks.
• The duration of prophylaxis depends on the severity of heart involvement, ranging from several years to lifelong treatment in severe cases.

★ Regular Medical Check-ups:

• Routine check-ups with a healthcare provider help monitor heart health and detect any early signs of complications.
• Patients with heart involvement may need periodic echocardiograms to assess heart valve function.

★ Education and Awareness:

• Raising awareness among healthcare providers, parents, and communities about the importance of early diagnosis and treatment of strep throat can help reduce rheumatic fever cases.
• School-based health programs can promote hygiene practices and early reporting of sore throat symptoms.

Tertiary Prevention:

For individuals who develop complications such as rheumatic heart disease (RHD), tertiary prevention focuses on managing symptoms and preventing further deterioration. This includes:

◆ Medical Management:

• Use of medications such as anti-inflammatory drugs, diuretics, and beta-blockers to manage symptoms.
• Regular follow-ups with cardiologists for individuals with RHD.

◆ Surgical Interventions:

• In severe cases, heart valve repair or replacement surgery may be necessary to restore normal heart function.

Conclusion:

Rheumatic fever is a serious but preventable disease. Early diagnosis and treatment of strep throat can significantly reduce the risk of developing this condition. Awareness, timely medical intervention, and proper hygiene practices are key to preventing its complications, particularly rheumatic heart disease. If you suspect a strep infection, seeking medical care promptly can help protect long-term health.